Unusual types of dementia: Corticobasal degeneration

Published: Tuesday, 06 September 2016

Dr Dan Nightingale continues our series on unusual types of dementia and provides a case study of corticobasal degeneration.

Summary

  • Corticobasal degeneration (CBD) is a progressive neurological disorder characterised by nerve cell loss and atrophy of the brain.
  • Initial symptoms may first appear on one side of the body but eventually affect both sides.
  • Symptoms are similar to Parkinson’s disease, and include poor coordination, an absence of movements, rigidity, impaired balance and abnormal muscle postures.
  • There is no known cure, but there are many ways to manage symptoms and enhance quality of life.

There are over 100 types and subtypes of dementia, which are classified in a number of different ways. These classifications are used to group disorders that have some features in common. They are:

  • Cortical dementia – the brain damage primarily affects the brain’s cortex, or outer layer. Cortical dementias tend to cause problems with memory, language, thinking and social behaviour.
  • Subcortical dementia – affects parts of the brain below the cortex. Subcortical dementia tends to cause changes in emotions and movement in addition to problems with memory.
  • Progressive dementia – gets worse over time, gradually interfering with more and more cognitive abilities.
  • Primary dementia – dementia such as Alzheimer’s disease (AD) that does not result from any other disease.
  • Secondary dementia – occurs as a result of a physical disease or injury.

The correct term for any type of dementia now is neurocognitive disorder. So, for example, it is neurocognitive disorder due to Alzheimer’s disease.

In this article, I will discuss my patient Carole (not her real name), who was diagnosed with corticobasal degeneration (CBD).

Definition of CBD

The National Institute of Neurological Disorders and Stroke defines CBD as: ‘…a progressive neurological disorder characterised by nerve cell loss and atrophy (shrinkage) of multiple areas of the brain including the cerebral cortex and the basal ganglia.’ CBD progresses gradually. Symptoms typically begin around age 60. They may first appear on one side of the body, but eventually affect both sides as the disease progresses.

Symptoms are similar to those found in Parkinson’s disease. They include:

  • poor coordination
  • akinesia (an absence of movements)
  • rigidity (a resistance to imposed movements)
  • disequilibrium (impaired balance)
  • limb dystonia (abnormal muscle postures).

An individual with CBD may experience other symptoms, such as cognitive and visual-spatial impairments, apraxia (loss of the ability to make familiar, purposeful movements), hesitant and halting speech, myoclonus (muscular jerks), and dysphagia (difficulty swallowing). Eventually they become unable to walk.

Case study

Carole’s first hint that something wasn’t right occurred when she was walking back from the shop one day. She was having a hard time walking and thought it was sciatica. She barely made it home and had to stop and rest frequently. However, Carole isn’t one to give up, and eventually she got home and spent the rest of the day sleeping.

She went to see her GP who referred her to a neurologist. It was a few weeks before she got her appointment. As she waited, she noticed that her handwriting was getting smaller and smaller. In addition, she developed a limp in her left leg. On visiting the neurologist, he diagnosed her as having corticobasal degeneration.

The next two years flew by. The disease slowly progressed until she had limited use of her entire right side and painful rigidity and jerks/tremors. Carole is no longer able to work. The neurologist prescribed Sinemet. Her physical pain has improved about 50% and she has found that drinking chamomile tea eases tremors and rigidity, as well helping her with insomnia.

The AAA approach

The AAA approach relates to three components of supporting someone with a rare form of dementia, and this is the strategy I am using with Carole:

  • Assess
  • Advice
  • Act.

1. Assess
Assess every aspect of the individual. Get to know the person – and this goes way beyond carrying out a Roper, Logan and Tierney assessment or similar clinical tool. It incorporates really getting to know the personality and character of each individual. Focus on the positive aspects of their life, as this is what will help build a relationship. Then look at the challenges. Prioritise them – explore exactly what the biggest challenge is for the individual. Make use of the identified strengths to compensate for identified challenges.

2. Advice
Advice can be sought from all members of the multi-disciplinary professions. These include: GP; psychotherapist; psychologist; psychotherapist; admiral nurse; social worker; speech and language therapist, and anyone else involved.

Don’t forget to include family members and friends. Remember to always have the individual at the centre of everything that is happening, to be involved with all discussions and decisions – for that is the definition of true person-centred care.

3. Act
Act by developing a shared action plan where outcomes of care are agreed by all those involved. Again, the individual living with dementia takes the lead role. Always bear in mind the Mental Capacity Act 2005 (England) and the Mental Incapacity Act (Scotland) when the individual is making decisions. Always act in the best interests of the person you are supporting.

Treating Carole with hypno-psychotherapy

Carole agreed to undergo a six-week course of treatment using hypno-psychotherapy. Our aims were twofold:

  • help to reduce her anxiety and fear based around an uncertain future – there is no cure for CBD, so managing symptoms and improving quality of life were our main goals
  • improve sleeping, as she had trouble falling asleep (though once asleep, she would not wake for a good six hours).

I saw Carole in my office for approximately 90 minutes each week for six weeks. By using a six-stage process of hypno-psychotherapy, we achieved our goals. I now see her every three months for follow-up. The six-stage process was as follows:

  1. eye closure
  2. progressive muscle relaxation
  3. a trigger (this is a word used to encourage deep trance)
  4. a deepener (this is a technique that takes the person into a deep state of hypnosis)
  5. treatment/therapy (through guided visual imagery, Carole left my office and entered a forest) – through direct suggestion, I made positive post-hypnotic suggestions to her subconscious mind)
  6. termination (this is where I orientated Carole back to the present).

It’s always important to remember that if there is no known cure, there are many ways in which we can manage symptoms and enhance quality of life. I have now prescribed gentle exercise, adult colouring and cognitive stimulation therapy so that Carole has a positive focus. I have also taught Carole, and her best friend, self-hypnosis. This means she can drift off to her special place (forest) at any time she chooses. There she can find peace and calmness and reduce any feelings of anxiety that might surface.

Further information

About the author

Dr Daniel Nightingale is a clinical dementia specialist, speaker and author. This email address is being protected from spambots. You need JavaScript enabled to view it.; www.dementiatherapyspecialists.com

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